• Barry J Maron, Paolo Spirito, Michael J Ackerman, Susan A Casey, Christopher Semsarian, N A Mark Estes, Kevin M Shannon, Euan A Ashley, Sharlene M Day, Giuseppe Pacileo, Francesco Formisano, Emmanuela Devoto, Aristidis Anastasakis, J Martijn Bos, Anna Woo, Camillo Autore, Robert H Pass, Giuseppe Boriani, Ross F Garberich, Adrian K Almquist, Mark W Russell, Luca Boni, Stuart Berger, Martin S Maron, and Mark S Link.
• Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN 55407, USA. hcm.maron@mhif.org
• J. Am. Coll. Cardiol. 2013 Apr 9; 61 (14): 1527-35.

ObjectivesThe aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM).BackgroundHCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients.MethodsA multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia.ResultsDefibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age.ConclusionsIn a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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